primary sarcoma of left atrium - bmj

Brit. HeartJ., 1966, 28, 287.

Primary Sarcoma of the Left AtriumE. B. RAFTERY, S. AHMED, and M. V. BRAIMBRIDGE

From the Cardiac Department and Cardiac Surgical Unit, St. Thomas's Hospital, London S.E.1

Primary tumours of the heart are rare (Yater,1931) and can present difficult diagnostic problems.Not only do they simulate valvular disease of theheart, but they are sometimes accompanied by anon-specific syndrome of fever, anemia, clubbingof the fingers and toes, hemoptysis, and a raisederythrocyte sedimentation rate (ESR). This maybe very difficult to distinguish from subacute bac-terial endocarditis, pulmonary tuberculosis, or car-cinoma ofthe bronchus, and has only been describedin association with benign myxomas of the leftatrium (Goodwin et al., 1962). This report is of aleft atrial sarcoma which presented in this way.

Case ReportThe patient, a 53-year-old lorry driver, presented in

May 1963 with a two-week history of exertional dyspneeaand ankle swelling. There was no past history of rheu-matic fever or chorea, and he had always been in goodhealth before the onset of these symptoms. On ex-amination, he was wasted, and in congestive heartfailure. The pulse was in sinus rhythm, with a rate of100 a minute, and a blood pressure of 150/80 mm. Hg.There was marked clubbing of the fingers and toes whichthe patient insisted was of recent appearance, but therewere no splinter hemorrhages or splenomegaly. Therewas clinical evidence of both right and left ventricularhypertrophy. On auscultation, there was a loud andwidely split first heart sound followed by a pansystolicmurmur. The pulmonary component of the secondheart sound was accentuated, and was followed by asoft opening snap. A short mid-diastolic murmur wasaudible at the apex (Fig. 1). The electrocardiogramwas within normal limits and the chest radiographshowed enlargement of the heart, particularly the leftatrium, and pulmonary cedema. At this time the hmemo-globin was 98 per cent, with a white count of 9,000 (nor-mal differential) and an ESR of 40 mm. (Westergren).Blood cultures were negative.He made an excellent symptomatic response to digi-

talis and diuretic therapy, and returned to work afterthree weeks. He presented again one month later insevere congestive heart failure, with a cough, briskhemoptysis, and a low-grade fever. A large right-

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sided pleural effusion had developed and the electro-cardiogram showed a shift of axis to the right. Themid-diastolic murmur was less obvious than on hisprevious admission, but could not be made to vary withpositional changes. The hemoglobin was 90 per centwith a white cell count of 9,150 (64% neutrophils, 15%lymphocytes, 15% eosinophils, 6% monocytes) and anESR of 35 mm. Six blood cultures were negative, andno pathogens or malignant cells could be found innumerous specimens of sputum. Pleural aspiration re-vealed a fluid with the characteristics of a transudate, con-taining only a few mesothelial cells and lymphocytes.The total serum proteins were 6-6 g./100 ml. (albumin5-1 g., globulin 1-5 g.), and electrophoresis showed onlya small increase in the a-globulin level. Six lupuserythematosus cell preparations were negative. Tomo-grams of the chest failed to reveal any localized lesion,and bronchoscopy revealed only slight cedema of thebronchial mucous membrane.

Despite a six-week course of intramuscular penicillin(six million units daily), the fever and haemoptysis per-sisted. He responded only slowly to intensive diuretictherapy, and the haemoglobin fell steadily to 53 per centwith a serum iron of 53 ug./100 ml. The sternal mar-row cells showed gross iron deficiency. The ancmiaresponded promptly to a course of intravenous iron.The white cell count continued to be within normallimits except for a slight eosinophilia ranging from 4 to15 per cent. The ESR varied from 12 to 40 mm.

Cardiac catheterization in January 1964 showed themean pulmonary wedge pressure to be 25 mm. Hg andthe pulmonary artery pressure to be 56/28 mm. Hg.Selective pulmonary angiography revealed marked nar-rowing of the small pulmonary arteries, but the leftatrium failed to opacify despite prolonged observation.In spite of this, a clinical diagnosis of left atrial myxomawas made, and the patient was offered the opportunityof surgery. He refused, and returned home. Twomonths later he presented again in severe congestivefailure, and after a further period of intensive treatmentwas submitted to thoracotomy.

Operation (July 7). Before the thorax was opened, asupportive bypass from the right atrium to femoralartery was made ready to drain the atrium through acatheter inserted through the femoral vein. Rightatrial

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Raftery, Ahmed, and Braimbridge

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FIG. 1.-Phonocardiograms before (A) and after (B) removal of the tumour. Note that the softopening snap and mid-diastolic murmur in (A) are not evident in (B). PA(HF), pulmonary area,high frequency; MA(LF), mitral area, low frequency; MA(HF), mitral area, high frequency; S1,first heart sound; P2, pulmonary component of the second heart sound; MDM, mid-diastolic

murmur.

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Primary Sarcoma of the Left Atrium

blood could be passed through the Osborn-Bramson-Gerbode oxygenator and returned to the femoral artery.This technique was necessary because of the poor con-dition of the patient, which, it was felt, would deterioratefurther during cardiac manipulation.A left thoracotomy was performed through the 5th

intercostal space dividing the sternum. The lung wasnot stiff and there was no septal cedema or left pleuralfluid; 1,200 ml. were aspirated from the right pleura.A firm mass in the right transverse fissure was thoughtto be an encysted effusion. The pericardium wasopened and the left atrium was shown to be muchenlarged.No pressure measurements were made from the left

side of the heart because of the danger of dislodgingtumour. The blood pressure slowly deteriorated andthe venous oxygen saturation fell. Femoro-femoral by-pass was, therefore, begun, with immediate improve-ment in blood pressure and venous oxygen saturation,and the heart was explored. A firm sessile tumour wasfelt on the medial wall of the left atrium, attached to thevalve ring. There was moderate mitral incompetence.A cannula was inserted in the right atrial appendage

and joined to a Y piece in the femoral venous line. By-pass was made total without interruption, a left ventricu-lar vent was inserted and the heart was fibrillated elec-trically. The left atrium was opened and the tumourwas seen as a firm, yellow, sessile mass, 6 by 3 cm. indiameter, quite unlike a myxoma, extending downthrough the valve ring which had made a waist on thetumour by compression. The base of the tumour wasattached to the medial part of the mitral valve ring abovethe commissure over an area 2-5 by 1-5 cm. The valvecusps were normal. There was a second small tumour,1 cm. in diameter, in the orifice of the left inferior pul-monary vein.

Both tumours were removed as far as possible and their

bases scraped. Total removal was obviously imprac-ticable. The left atrium was closed, the heart restartedwith a single D.C. shock and bypass was discontinued.The chest was closed with pleural drainage and a trache-ostomy performed for artificial respiration.

Post-operative Course. The patient's post-operativeprogress was slow but uninterrupted. Artificial respira-tion was discontinued after five days.

Radiotherapy was given in the Radiotherapy Depart-ment of St. Thomas's Hospital with cobalt 60 tele-therapy to left atrium and upper mediastinum. Atumour dose of 5,000 r. was given in 12 treatments over28 days.When seen in the out-patient department in October

1964, he was considerably improved though still withsome cough and effort intolerance. He was in sinusrhythm, with a jugular venous pressure of 2 cm.; onauscultation there was an early systolic murmur but theopening snap had disappeared (Fig. 1). He was re-catheterized in March 1965, when the mean pulmonarywedge pressure was 3 mm. Hg.A large opacity had appeared at the right hilum and a

smaller one at the left apex. He was given a secondcourse of radiotherapy to these opacities, but slowlydeteriorated and died of bronchopneumonia without anyclinical evidence of heart failure. At necropsy, theheart was found to be completely free of tumour, thougha little scarring could be seen on the medial wall of theleft atrium. Large secondary deposits were present inboth lungs, but there was no obvious tumour in thepulmonary veins or arteries.

Pathological Specimen. The main tumour was 6 by3 cm. with a central waist. The second tumour was 1cm. in diameter. Histologically, both were undifferen-tiated sarcomata with numerous large strap-like cells,some multinucleated, invading the atrial wall (Fig. 2).

i

...4...8 ...... v.~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~..........

FIG. 2.-Macroscopical and microscopical appearances of the tumour. Note the pleomorphic appearance with large numbersof strap-like cells. Special staining failed to produce any evidence of cross-striation.

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There was some endocardial thrombus overlying partof the growth, but no evidence of a rhabdomyosarcoma,special stains failing to reveal any cross-striations. Theappearances suggested a primary rather than a secondarygrowth because of the diffuse infiltration of the heartwall.

DiscussionThis case report illustrates the difficulty which

may be experienced in diagnosing cardiac tumours.The auscultatory sign most commonly associatedwith tumours is a mid-diastolic murmur whichvaries with positional changes and simulates mitralstenosis. In this case the signs were suggestive ofmitral incompetence (Wittenstein et al., 1959), andthe murmur did not vary because the tumour wasfixed in the valve orifice. The origin of the "open-ing snap " is not clear, but it has been suggested thatit is produced by the movement of the tumour dur-ing atrial systole (Abbott, Warshawski, and Cobbs,1962), and the fact that it disappears after thetumour is removed supports this suggestion.

Malignant cardiac tumours usually present withthe physical signs of pericardial effusion, and thediagnosis is most commonly made by examinationof aspirated fluid (Johnson and Stokes, 1964;Zemansky, 1928). This is clearly a function of theway in which the tumour spreads, and previousnecropsy reports confirm that most grow into thepericardium and not into the atrial cavity (Yater,1931). Pascuzzi et al. (1957) described a rhabdo-myosarcoma which encroached upon the atrialcavity and was associated with hypertrophic osteo-arthropathy. No other case of malignant tumourhas been reported as having any symptoms of sys-temic disease. It seems likely that this syndromemay occur with any tumour that encroaches uponthe chambers of the heart, but it has been associatedparticularly with myxomas because they are com-moner than the exceedingly rare primary malignanttumours. Furthermore, the rapid growth of malig-

nant tumours makes early pericardial involvementand death more common than valve obstruction andthe systemic symptoms which seem to be associatedwith it.

SummaryA case is reported of primary sarcoma of the left

atrium presenting as mitral incompetence and in-tractable heart failure. The tumour was associatedwith fever, anamia, clubbing of fingers and toes,and a raised ESR, a syndrome that has previouslybeen described in association only with benignprimary tumours. The tumour was partially re-moved with the aid of extracorporeal circulation,and the residue was treated with radiotherapy. Atnecropsy there was no macroscopic evidence of theprimary tumour, but large secondary deposits werepresent in both lungs.

Thanks are due to Dr. Raymond Daley for permissionto publish this case.

ReferencesAbbott, 0. A., Warshawski, F. E., and Cobbs, B. W. (1962).

Primary tumors and pseudotumors of the heart. Ann.Surg., 155, 855.

Goodwin, J. G., Stanfield, C. A., Steiner, R. E., Bentall, H.H., Sayed, H. M., Bloom, V. R., and Bishop, M. B.(1962). Clinical features of left atrial myxoma.Thorax, 17, 91.

Johnson, A. G., and Stokes, J. F. (1964). Fibrosarcoma of theheart diagnosed during life. Brit. med. J., 1, 480.

Pascuzzi, C. A., Parkin, T. W., Bruwer, A. J., and Edwards,J. E. (1957). Hypertrophic osteoarthropathy associ-ated with primary rhabdomyosarcoma of the heart.Proc. Mayo Clin., 32, 30.

Wittenstein, G. J., Grow, J. B., Hoffman, M. S., Gensini,G. G., and Denst, J. (1959). "Myxoma" of the leftatrium simulating pure mitral insufficiency. Surgery,45, 981.

Yater, W. M. (1931). Tumors of the heart and pericardium.Arch. intern. Med., 48, 627.

Zemansky, A. P. (1928). The examination of fluids for tumorcells. Amer. J. med. Sci., 175, 489.

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